Corneal Dystrophy, Subepithelial Mucinous

Clinical Characteristics
Ocular Features: 

This disorder, reported so far in a single family, is an anterior corneal dystrophy with onset in the first decade of life.  The frequency of epithelial erosions tended to subside during adolescence but visual acuity continued to decline secondary to subepithelial nodular opacities and a generalized haze most dense centrally. No geographic lines are present and cystic changes in the epithelium were absent.  Bowman layer and deeper stuctures of the cornea are unaffected. Patients may have 20/30 vision into the fifth decade but after that it may decrease into the 20/400 range.  EM revealed accumulations of subepithelial fibrillar material.  Light microscopy and immunohistochemistry showed the material to be chondroitin-4-sulfate and dermatan sulfate.

Systemic Features: 

No systemic disease association has been reported.


In the single 3 generation family reported, the pattern of inheritance was consistent with autosomal dominant inheritance.  No locus or mutation has been reported.

Treatment Options: 

The usual treatment for acute corneal erosions might be beneficial but other treatments have not been reported.  Penetrating keratoplasty and superficial keratectomy have been used on several patients but followup is not available.

Article Title: 


Feder RS, Jay M, Yue BY, Stock EL, O'Grady RB, Roth SI. Subepithelial mucinous corneal dystrophy. Clinical and pathological correlations. Arch Ophthalmol. 1993 Aug;111(8):1106-14.

PubMedID: 8352693